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Journal Articles Orphanet Journal of Rare Diseases Year : 2014

Ethnicity and association with disease manifestations and mortality in Behçet's disease

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Abstract

Background
Behçet's disease (BD) significantly increases morbidity and mortality. BD mainly affects young adults with a peculiar geographical distribution. It has been suggested that BD varies in its phenotypic expression in different ethnic groups.
Methods
We investigated potential ethnicity-related differences relative to phenotype and prognosis of BD patients in a French multiethnic country. We included 769 consecutive patients fulfilling the international criteria of classification for BD, in the 3 largest ethnic groups of our cohort [European (n = 369), North African (n = 350) and sub Saharan African (n = 50)]. Factors that affect prognosis were assessed by multivariate analysis.
Results
535 (69.6%) patients were male and the median (IQR) age at diagnosis was of 30.9 (24.9-37.2) years. Sub Saharan African BD patients had a higher frequency of CNS involvement (48% vs 32.3% vs 29.5%, p = 0 .035), a higher rate of death (12% vs 6% vs 3.5%, p = 0.029) and a lower frequency of HLA B51 allele (29.4% vs 49.2% vs 55.8%, p = 0.009) compared to those from North Africa and Europe, respectively. Multivariate analysis showed that male gender (HR: 5.01, CI: 1.51-16.65), cardiovascular involvement (HR: 2.24, CI: 1.15-4.36), and sub Saharan African origin (HR 2.62 (0.98-6.97) were independently associated with mortality. The 15-year mortality rate was of 19%, 9% and 6% in sub Saharan African, North African and European BD patients, respectively (p = 0.015).
Conclusion
We reported ethnicity-related differences with respect to phenotype of BD. Sub Saharan Africans patients exhibited a worse prognosis.
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Dates and versions

inserm-00981306 , version 1 (22-04-2014)

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Lea Savey, Mathieu Resche-Rigon, Bertrand Wechsler, Cloé Comarmond, Jean Charles Piette, et al.. Ethnicity and association with disease manifestations and mortality in Behçet's disease. Orphanet Journal of Rare Diseases, 2014, 9 (1), pp.42. ⟨10.1186/1750-1172-9-42⟩. ⟨inserm-00981306⟩
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